Sam Mehr

Food Protein-Induced Enterocolitis Syndrome: 16-Year Experience

Objective. The goal was to examine the demographic characteristics, causative foods, clinical features, treatments, and outcomes for children presenting with acute food protein-induced enterocolitis syndrome. Methods. This was a retrospective study of children with food protein-induced enterocolitis syndrome who presented to the Childrens Hospital at Westmead (Sydney, Australia) over 16 years. Results. Thirty-five children experienced 66 episodes of food protein-induced enterocolitis syndrome. The mean age at initial presentation was 5.5 months. Children frequently experienced multiple episodes before a correct diagnosis was made. Twenty-nine children reacted to 1 food, and 6 reacted to 2 foods. Causative foods for the 35 children were rice (n = 14), soy (n = 12), cows milk (n = 7), vegetables and fruits (n = 3), meats (n = 2), oats (n = 2), and fish (n = 1). In the 66 episodes, vomiting was the most common clinical feature (100%), followed by lethargy (85%), pallor (67%), and diarrhea (24%). A temperature of <36°C at presentation was recorded for 24% of episodes. A platelet count of >500 × 109 cells per L was recorded for 63% of episodes with blood count results. Only 2 of the 19 children who presented to an emergency department with their initial reactions were discharged with correct diagnoses. Additional investigations of food protein-induced enterocolitis syndrome episodes presenting to the hospital were common, with 34% of patients undergoing abdominal imaging, 28% undergoing a septic evaluation, and 22% having a surgical consultation. Prognosis was good, with high rates of resolution for the 2 most common food triggers (ie, rice and soy) by 3 years of age. Conclusions. Misdiagnosis and delays in diagnosis for children with food protein-induced enterocolitis syndrome were common, leading many children to undergo unnecessary, often painful investigations. Decreased body temperature and thrombocytosis emerge as additional features of the syndrome.

Paediatric anaphylaxis: a 5 year retrospective review

Objective:  To describe the demographic characteristics, clinical features, causative agents, settings and administered therapy in children presenting with anaphylaxis.

Non–IgE-mediated gastrointestinal food allergy

Non-IgE-mediated gastrointestinal food-induced allergic disorders (non-IgE-GI-FAs) account for an unknown proportion of food allergies and include food protein-induced enterocolitis syndrome (FPIES), food protein-induced allergic proctocolitis (FPIAP), and food protein-induced enteropathy (FPE). Non-IgE-GI-FAs are separate clinical entities but have many overlapping clinical and histologic features among themselves and with eosinophilic gastroenteropathies. Over the past decade, FPIES has emerged as the most actively studied non-IgE-GI-FA, potentially because of acute and distinct clinical features. FPIAP remains among the common causes of rectal bleeding in infants, while classic infantile FPE is rarely diagnosed. The overall most common allergens are cows milk and soy; in patients with FPIES, rice and oat are also common. The most prominent clinical features of FPIES are repetitive emesis, pallor, and lethargy; chronic FPIES can lead to failure to thrive. FPIAP manifests with bloody stools in well-appearing young breast-fed or formula-fed infants. Features of FPE are nonbloody diarrhea, malabsorption, protein-losing enteropathy, hypoalbuminemia, and failure to thrive. Non-IgE-GI-FAs have a favorable prognosis; the majority resolve by 1 year in patients with FPIAP, 1 to 3 years in patients with FPE, and 1 to 5 years in patients with FPIES, with significant differences regarding specific foods. There is an urgent need to better define the natural history of FPIES and the pathophysiology of non-IgE-GI-FAs to develop biomarkers and novel therapies.

Doctor – How do I use my EpiPen?

Parents and children who have been prescribed an Epipen are often unable to demonstrate its correct administration. One contributory factor may be that doctors are unfamiliar with the EpiPen and are unable to demonstrate the correct administration of the pen to the family. The aim of this study was to determine the rate of correct EpiPen demonstration by junior and Senior Medical Staff at a major tertiary paediatric Hospital. Junior and Senior medical staff were scored on their ability to correctly use the EpiPen trainer. A 6 step scoring system was used. One‐hundred doctors were recruited (Residents n = 31, Senior Residents n = 39, Fellow/Consultants n = 30). Junior and Senior Medical staff had similar scores for EpiPen demonstration, the number that needed to read the EpiPen instructions prior to use and the frequancy of accidental self‐injection into the thumb. Only two doctors (2%) demonstrated all 6 administration steps correctly. The most frequent errors made were not holding the pen in place for >5 seconds (57%), failure to apply pressure to activate (21%), and self‐injection into the thumb (16%). Ninety five doctors needed to read the instructions, and of these, only 39 (41%) then proceeded to correctly demonstrate the remaining 5 steps. Forty‐five doctors had previously dispensed an EpiPen, but only three demonstrated its use to parents/children with a trainer. The majority of doctors do not know how to use an Epipen and are unable to provide appropriate education to parents/children. In 37% of cases, the demonstration would not have delivered adrenaline to a patient.

Streptococcus pneumoniae – a review of carriage, infection, serotype replacement and vaccination

Invasive pneumococcal infection remains a leading global cause of morbidity and mortality in young children. In developed nations, a substantial decrease in the incidence of IPD has been achieved with inclusion of the 7 valent protein conjugated pneumococcal vaccines (7vPCV) into paediatric vaccine schedules. In contrast, the incidence of IPD has changed little in developing nations. This is likely due to poor access to medical care and pneumococcal vaccination, the accompanying HIV and malnutrition burden, and the fact that 7vPCV does not contain the most common serotypes (1,5, 6A) responsible for IPD in many developing nations. The battle against IPD in developed nations is not over, with the rise of non-7vPCV serotypes since routine 7vPCV vaccination. This has necessitated the development and distribution of pneumococcal vaccines containing 3 or 6 additional serotypes. This article provides an overview on pneumococcal carriage and risk factors for IPD, the rise of non-7vCPV serotypes in the era of 7vPCV vaccination, and the current and newly available broader valent pneumococcal vaccines.

Rice: a common and severe cause of food protein induced enterocolitis syndrome.

Objective: To examine and compare the characteristics of food protein-induced enterocolitis syndrome (FPIES) caused by rice and cow’s milk/soy. Design: Retrospective study of children presenting with FPIES to the Children’s Hospital at Westmead, NSW, Australia, over a 16-year period. Results: There were 14 children with 26 episodes of rice FPIES compared with 17 children with 30 episodes of cow’s milk (n = 10) or soy (n = 7) FPIES. Children with rice FPIES were more likely to have FPIES caused by other foods (36%) than children with FPIES caused by cow’s milk/soy (0%). Rice caused more episodes of FPIES before a correct diagnosis was made (median 4 (range 1–4) vs median 2 (range 1–4)) and triggered more severe reactions with higher rates of intravenous fluid resuscitation (42% vs 17%) than reactions caused by cow’s milk/soy. Conclusions: This study highlights the emerging importance of rice, a food commonly thought to be “hypoallergenic”, as a significant trigger of FPIES. Paediatricians should be aware that rice not only has the potential to cause FPIES, but that such reactions tend be more severe than those caused by cow’s milk/soy.

Allergic Reactions to Propofol in Egg-Allergic Children

BACKGROUND: Egg and/or soy allergy are often cited as contraindications to propofol administration. Our aim was to determine whether children with an immunoglobulin (Ig)E-mediated egg and/or soy allergy had an allergic reaction after propofol use. METHODS: We performed a retrospective case review over an 11-year period (1999–2010) of children with IgE-mediated egg and/or soy allergy who had propofol administered to them at the Childrens Hospital Westmead, Sydney. RESULTS: Twenty-eight egg-allergic patients with 43 propofol administrations were identified. No child with a soy allergy who had propofol was identified. Twenty-one children (75%) were male, the median age at anesthesia was 2.4 years (range, 1–15 years), and the presence of other atopic disease was common (eczema 61%, asthma 32%, peanut allergy 43%). Most children (n = 19, 68%) had a history of an IgE-mediated clinical reaction to egg with evidence of a significantly positive egg white skin prick test (SPT) reaction (≥7 mm). Two of these had a history of egg anaphylaxis. The remaining children (n = 9, 32%) had never ingested egg because of significantly positive SPT (≥7 mm). All SPTs to egg were performed within 12 months of propofol administration. There was one nonanaphylactic immediate allergic reaction (n = 1 of 43, 2%) that occurred 15 minutes after propofol administration in a 7-year-old boy with a history of egg anaphylaxis and multiple other IgE-mediated food allergies (cows milk, nut, and sesame). SPT to propofol was positive at 3 mm. No other egg-allergic child reacted to propofol. CONCLUSIONS: Despite current Australian labeling warnings, propofol was frequently administered to egg-allergic children. Propofol is likely to be safe in the majority of egg-allergic children who do not have a history of egg anaphylaxis.

Clinical predictors for biphasic reactions in children presenting with anaphylaxis.

Background One of the main reasons for hospital admission once a child has been stabilized following anaphylaxis is to monitor for a biphasic reaction. However, only a small percentage of anaphylactic episodes involve biphasic reactions that would benefit from admission. Identification of predictive factors for a biphasic reaction would assist in determining who may benefit from prolonged observation.

Safety of food challenges to extensively heated egg in egg-allergic children: a prospective cohort study

Many children with IgE‐mediated allergy to egg can tolerate egg in baked foods. However, the clinical characteristics and severity of reactions of egg‐allergic children who react to baked egg at open food challenge (OFC) are not well defined.

CHARGE syndrome: a review.

CHARGE syndrome is a complex genetic syndrome, owing to the wide range of tissues/systems affected by mutations in the CHD7 gene. In this review, we discuss the diagnosis, clinical features and management of CHARGE syndrome.